DLBCL Flashcards
Hans Method - DLBCL subtypes:
-Step 3
MUM1
- Positive, then Non-GBC (ABC)
- Negative, then GBC
What is true about late (>5 years) PTLD?
- EBV Negative
- More Aggressive
Posttransplant Lymphoproliferative DIsorder (PTLD):
- When do they most often occur?
- What are the “heralded” by?
- <1 year post-transplant
- Heralded by elevated EBV-DNA
Lymphomatoid Granulomatosis most commonly affects which organs? (4)
-What is it associated with?
-Respiratory
-Brain
-Kidney
-Liver
Associated with EBV and Immunodeficiency
DLBCL clinical presentation.
- Rapidly enlarging lymph node or extranodal site
- Localized at presentation
*BM involvement uncommon (10%)
ALK+ DLBCL:
- Neoplastic cells
- Nodal or Extranodal
- HIV or EBV association
- Immunoblastic/Plasmablastic that express ALK
- Nodal
- NOT ass. w/ HIV or EBV
What are the most common lymphomas in HIV? (5)
- Burkitt Lymphoma
- DLBCL (CNS)
- Primary Effusion Lymphoma
- Plasmablastic Lymphoma
- Hodgkin Lymphoma
T-Cell/Histiocyte Rich DLBCL (TCRBCL):
-Small lymphocyte IHC (+2;-3)
Positive: -Mixture of CD4+ and CD8+ Negative: -CD57 -CD21 -CD23 *NO T-cell rosettes or small B cells
Primary Mediastinal (Thymic) Large B-cell Lymphoma:
- Age
- Morphology
- Adult Females (F:M;2:1)
- Sclerosing lymphoma with large B cells entrapped within bands of sclerosis
Primary Mediastinal (Thymic) Large B-cell Lymphoma: -IHC (+5;-3)
Positive: -CD19 -CD20 -CD30 -CD45 -CD79a Negative: -CD5 -CD10 -Surface Igs
Hans Method - DLBCL subtypes:
-Step 1
CD10
- Positive (>30%), then GCB
- Negative, step 2
Ki67 expression in DLBCL.
60-99%
Which type of DLBCL, Germinal Center (GCB) or Activated B-cell (ABC; nonGCB), has a better response to treatment?
Germinal Center (GCB) type
TCRBCL Bone Marrow involvement pattern.
Paratrabecular lymphoid aggregates
T-Cell/Histiocyte Rich DLBCL (TCRBCL):
- Age
- Morphology
T-Cell/Histiocyte Rich DLBCL (TCRBCL):
- 40 y/o (mean; kids-elderly)
- Diffuse proliferation of small lymphocytes and histiocytes with scattered large B cells
Primary Mediastinal (Thymic) Large B-cell Lymphoma: -Molecular/Cytogenetics
Alterations in the MAL gene
-Gains in 9p (location of JAK2)
*NO rearrangement of BCL2 or BCL6
Rearrangement of BCL6 is more common in what type of DLBCL?
Non-Germinal Center (ABC) type
CD5 expressing DLBCL must be distinguished from what?
Blastoid MCL (bcl-1+)
What are the most common Primary Immunodeficiencies (PID) with Lymphoproliferative disorders (LPD)? (5)
- Ataxia Telangiectasia
- Wiskott Aldrich syndrome
- Common Variable Immunodeficiency
- X-linked Lymphoproliferative disorder (Duncan disease)
- Nijmegan Chromosomal Breakage Syndrome
What gene rearrangements are present in 20-30% of DLBCL?
BCL2 and BCL6
Hans Method - DLBCL subtypes:
-Step 2
BCL6
- Negative, then Non-GBC (ABC)
- Positive, step 3
What is Primary Effusion Lymphoma (PEL) associated with?
HHV8 and HIV
DLBCL is what percentage of non-Hodgkin lymphomas?
30%
DLBCL IHC:
-Positive for (5)
- CD19
- CD20
- CD22
- CD45
- bcl-2
DLBCL IHC:
-Variable for (3)
- CD5
- CD10
- bcl-6
What rearrangement is more common in GCB-type DLBCL?
BCL2
-t(14;18)
Primary CNS DLBCL:
-Molecular/Cytogenetics
Most cases have BCL6 rearrangement
-Overexpress bcl-6
*BCL2 rearrangement is RARE
Intravascular Large B cell lymphoma is AKA (3).
- Symptoms due to what?
- Nodal or Extranodal
-Angioednotheliomatosis
-Angiotropic lymphoma
-Intravascular lymphomatosis
Small vessel obstruction by large B cells
-LN involvement is RARE
Where is the BCL6 gene located?
- What is it commonly rearranged with?
- Non-GCB (ABC) type or GCB type
3(q27)
- t(3;14)
- Non-GCB (ABC)
What is HHV8/HIV associated with? (3)
- PEL
- Castleman
- Kaposi
T-Cell/Histiocyte Rich DLBCL (TCRBCL):
-Large B cell IHC (+3;-3)
Positive: -pan B-cell -bcl-6 -EMA (+/-) Negative: -CD15 -CD30 -EBV
Where is the BCL2 gene located?
-Non-CGB (ABC) type or GCB type?
18(q21)
-GCB type
Lymphomatoid Granulomatosis (LG) morphology and cell types.
- Vasculitis pattern (Large Bs invade vessel wall)
- Large number of reactive T-cells, plasma cells, and histiocytes
- Granulomas UNcommon
PTLD Risk Factors:
- Renal/BMT recipients
- Heart-Lung/Liver-Bowel
- Age
- Pretransplant EBV status
- Degree of Immunosuppression
PTLD Risk Factors:
- Renal/BMT- Lower Risk
- Heart-Lung/Liver-Bowel - Higher Risk
- Age - Kids at Higher Risk
- Pretransplant EBV status - Negative
- Degree of Immunosuppression - Most suppressed; Highest risk
Plasmablastic DLBCL:
- Neoplastic cells
- Positive IHC (5)
- Negative IHC (3)
- Nodal or Extranodal
- HIV or EBV association
-Immunoblastic/Plasmablastic Positive IHC: -CD38 -CD138 -MUM1 (IRF4) -cytoplasmic Ig -EBV Negative IHC: (contrast to plasmacytoma) -CD20 -CD45 -CD56 Extranodal -mucosa of oral cavity Ass. w/ HIV and EBV
What are the most common Lymphoproliferative disorders (LPD) associated with Primary Immunodeficiencies (PID)? (3)
- DLBCL
- Lymphomatoid Granulomatosis
- T-Cell neoplasm
Primary Effusion Lymphoma IHC:
-Positive for (6)
- HHV8
- CD30
- CD45
- CD38
- CD138
- EMA
Primary CNS DLBCL:
- Age
- Radiographic
- May present or recur as
- Histology
Primary CNS DLBCL:
- 60 y/0
- Supratentorial mass (looks like GBM)
- Intraocular lymphoma
- Perivascular cuffs (express pan B cell antigens)
